Abstract #0964

Increased Coronary Vessel Wall Thickness in Hyper IgE Syndrome Patients; Depiction by Magnetic Resonance Vessel Wall Imaging and Pathological Correction

Khaled Z. Abd-Elmoniem¹, Nadine Z. Ramos¹, Saami Yazdani², Ahmed M. Ghanem^1,3, Steven M. Holland⁴, Alexandra F. Freeman⁴, and Ahmed M Gharib¹

¹Biomedical and Metabolic Imaging Branch, NIDDK, Bethesda, MD, United States, ²University of Southern Alabama, Mobile, AL, United States, ³Electrical Engineering, Suez Canal University, Ismailia, Egypt, ⁴NIAID, Bethesda, MD, United States

In this study, coronary wall MRI is used to assess the coronary wall thickness of patients with autosomal dominant hyper-IgE (AD-HIES) or Job's syndrome; a primary immunodeficiency caused by mutations in STAT3. Supported by post-mortem histology, MRI coronary wall of AD-HIES patients was thicker than in healthy subjects but comparable to CAD patients. These findings suggest that coronary arteries in Job’s syndrome are affected with atherosclerosis, contrary to prior beliefs and study findings. Direct histologic evaluation confirms the presence of atherosclerosis with lack of needed supportive adventitial thickening and elastic components. These findings suggest mechanisms for weakened vessel wall that may lead to coronary dilation and aneurysm in AD-HIES.

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